Far and Away: Living with Bleeding Disorders in Remote Areas

Families living in remote areas face unique challenges for bleeding disorders care. Learn how a few families have managed—and how local providers can help.
Author: Linda Marsa

When Mick Graham fell down the stairs in early May, his legs went numb, and he was in agonizing pain. His mother, Kristal, rushed him to the hospital near their home in Roundup, Montana, where he had a CT scan that revealed a hemangioma (a blood-filled, noncancerous growth) on his spine. To get a confirmatory MRI quickly, mother and son made the 50-mile drive to Billings the next day.

The neurologist at the hospital in Billings thought 15-year-old Mick, who has severe hemophilia A, was better off having the growth removed by a surgical team with expertise in the treatment of bleeding disorders. Medical professionals at the Hemophilia and Thrombosis Center at the University of Colorado Anschutz Medical Campus in Aurora, just outside Denver, have supervised Mick’s care since birth.

The Rocky Mountain Hemophilia & Bleeding Disorders Association, which is the nearest National Hemophilia Foundation chapter, helped defray the expenses for Mick and Kristal to fly to Denver the next morning for the emergency operation. Months later, however, Mick was still in terrible pain, and doctors determined that he needed another procedure. Because there was no time to arrange a flight, Kristal hopped in the car with Mick and drove 600 miles through the night, arriving on time nine hours later for the early morning appointment. “Doctors were worried the tumor could burst,” Kristal says.

Educating Local Providers

This was not the first time that Mick’s life has been saved by doctors far away in metro Denver, and it probably won’t be the last. Because the family lives in a remote town, Kristal, who has four other children and heads up her own shipping company with 19 employees, has to be organized and resourceful to make sure her child gets the care he needs.

“The Hemophilia and Thrombosis Center in Denver was my first call when I found out I was pregnant,” says Kristal, who has known she was a carrier since age 11. Providers there helped her set up an amniocentesis to confirm the baby’s bleeding disorder, so she could prepare for the delivery.

She’s made sure the local emergency room in Roundup and the pediatric hematologist’s office in Billings have an extensive file on Mick, who had his first bleed at 3 months old and learned how to infuse himself by age 6. Because his factor has a shortened half-life—the medicine is gone from his system in six hours—he needs infusions every other day, and Kristal has to make sure that he has plenty of medication on hand and that it’s stocked at the local ER in case of emergencies.

Kristal has found a great deal of help from the Rocky Mountain Hemophilia & Bleeding Disorders Association. “They provide a network of support where everyone gets together and meets other people who are dealing with the same things, allowing us to trade stories and give solutions,” she says.

Still, the challenges Kristal faces are similar to those of many other families who live in rural areas and must manage a bleeding disorder. They often contend with insurance companies that don’t know the urgency of treating bleeds or using certain medications, and with health systems that have little experience handling these disorders.

Overcoming Obstacles

That was certainly the experience of Sherianne Schow when she and her family relocated from a Kansas City, Kansas, suburb to Kalispell, Montana, in 2016, so her husband, Adam, a pediatric anesthesiologist, could help open a children’s hospital there. With three sons having hemophilia A, the Schows must be constantly vigilant.

Living in a town with fewer than 25,000 residents presented new obstacles for the family, which was accustomed to large cities with extensive infusion providers. The specialty pharmacy in Kansas City was “really on top of things,” Sherianne recalls. “If we had a bleed, they would have medicine on our doorstep by 5 p.m., and they sent a nurse along to train our kids to do their own infusions. Here in Montana, we’ve had to really work to get care.”

For the past four years, Sherianne has struggled with physicians and insurers who are unfamiliar with bleeding disorders. “We had to contend with a doctor hired by the insurance company to approve or disapprove of drugs who didn’t think our children needed a specific type of factor replacement—all he knew was this was expensive, so we didn’t need it,” she says. “The specialists here in Kalispell are excellent, but their hands are tied, and we couldn’t find pharmacies that would coordinate with the insurance carrier and the infusion service.”

The Schows eventually switched insurance providers, and they also recently signed up with the Denver-area Hemophilia and Thrombosis Center so their sons would have consistent care.

Unfortunately, the family’s experience is fairly typical, says Carrie Laborde, MD, who was a pediatric hematologist/oncologist at Kalispell Regional Healthcare for five years and treated the Schow boys. “Numerous children had trouble getting the product that they needed, insurance companies had a total lack of understanding, and parents were forced to file lots of appeals, write letters and call over and over to get what they needed,” says Laborde, who recently moved to Baylor Scott & White Medical Center in Temple, Texas. “There’s so much more potential to have worsened conditions or things that are not caught soon enough or treated properly in rural areas because a hematologist is so far away.”

Extending the Network of Care

Research consistently shows that adequate treatment to stop or prevent bleeding episodes not only improves outcomes but also reduces costs. In 1975, a national network of hemophilia treatment centers (HTCs) was created to provide an integrated model of multidisciplinary, comprehensive care to treat people with complex and rare inherited bleeding disorders.

HTC teams consist of a pediatric or adult hematologist, a nurse coordinator and a physical therapist and often include dentists, orthopedists, pharmacists, social workers and infectious disease specialists. Studies by the Centers for Disease Control and Prevention indicate that people with hemophilia who get care at HTCs are 40% less likely to be hospitalized and 40% less likely to die because of bleeding complications.

Other research has shown that regular prophylactic treatments to prevent bleeding episodes that start early, before age 4, preserve the ability to move joints properly.

But the majority of HTCs are concentrated east of the Mississippi River, and people living in sparsely populated states, like Wyoming, Nevada, North Dakota and Montana, often must travel hundreds of miles to get proper care, especially in emergencies.

“This is the situation that many patients encounter,” says Meera Chitlur, MD, a pediatric hematologist/oncologist at Children’s Hospital of Michigan, an HTC in Detroit. “Most local physicians may not have the expertise that is required to take care of them on a day-to-day basis.”

Many of the larger HTCs increase access to care by operating satellite clinics in rural areas—a team from the Hemophilia and Thrombosis Center flies from Denver to the Montana cities of Bozeman, Missoula, Billings and Helena as well as to South Dakota and Wyoming at least once a year—and by offering telephone or video counseling to people in remote or underserved areas.

“In the pandemic, we’ve found telehealth to be a wonderful supplement, and we’re going to use it a lot more frequently now that we’re used to having video chats,” says Marilyn Manco-Johnson, MD, a pediatric hematologist/oncologist and director of the Denver-area Hemophilia and Thrombosis Center. “We even have our physical therapists assessing joints on video chat.”

Perhaps out of necessity, people who live in rural areas are remarkably independent and resourceful, Manco-Johnson adds. “The children are aware and educated about what to do in case of emergencies,” she says. “I’ve been impressed with parents who always have a backup plan.”

Connect with a Hemophilia Treatment Center Near You

The national network of HTCs provides an integrated model of comprehensive care for people with bleeding disorders. Find your closest center at dbdgateway.cdc.gov/htcdirsearch.aspx.

How Families Can Get the Best Care for Their Children

It can be difficult for children with bleeding disorders to get comprehensive treatment when they live in remote areas. Here are some tips:

Have your child wear a medical ID at all times in case of an accident or emergency. The ID should include diagnosis, medication doses, and phone numbers for doctors and treatment centers.

Have a comprehensive plan of care established by a hemophilia treatment center (HTC) that includes working with local hematologists and primary care physicians for day-to-day management. HTCs are uniquely qualified to provide preventive care and empower patients, even when they live far away.

Gather information related to your child’s bleeding disorder and treatment plan. Describe the seriousness of bleeds, detail the steps to take in an emergency and list contact information for the nearest HTC. Give a copy to each of your child’s doctors and the local ER and hospital.

Keep an adequate supply of medication on hand and bring some with you to the ER in the event of an emergency. “Local hospitals only carry a few select products,” says Carrie Laborde, MD, a pediatric hematologist/oncologist. “If a child has a bleed and goes to the local ER, potentially there could be no factor for the child.”

Rely on your local National Hemophilia Foundation chapter, which keeps families connected through events and educational seminars and advocates for the bleeding disorders community.